Paris Trousseau Syndrome is a bleeding disorder in which there may not be enough platelets in the body (thrombocytopenia) and/or platelets may not work correctly. Platelets are cells in the blood that help stop bleeding after injury occurs. When bleeding starts (inside or outside of the body) platelets turn on and go to the site of the broken blood vessels where they clump together and stick to the blood vessel wall to form the beginning of a blood clot. Once platelets have turned on, they release chemicals that trigger other platelets to come to stick to the blood clot. In Paris Trousseau, platelets do not release the chemicals to trigger other clotting factors and/or do not get sticky and clump together. This can cause prolonged bleeding or bleeding that stops and then starts back up again.
Paris Trousseau syndrome often occurs in people that do not have chromosome 11. Chromosomes are tightly wrapped strings of DNA that hold genes that determine characteristics of that person: hair color, eye color, height, etc. Each parent gives their child 23 chromosomes, for a total of 46 pairs. Chromosome 11 is important for several reasons. One of these reasons is that chromosome 11 carries a gene that promotes the body to make platelets. People diagnosed with Jacobsen Syndrome should be tested for Paris Trousseau because they are missing chromosome 11.
- Increased bruising and/or bruises that take a long time to heal
- Prolonged bleeding from injuries
- Nosebleeds
- Bleeding gums
- Excessive/prolonged bleeding from a circumcision
- Excessive/prolonged bleeding from surgeries
- Heavy or prolonged menstrual bleeding
- Bleeding into the joints (Rare)
Diagnosis
When testing for Paris Trousseau Syndrome, a series of blood testing will need to be done and a detailed bleeding history will be taken. People who should be tested for Paris Trousseau Syndrome include:
- Someone diagnosed with Jacobsen Syndrome because they are missing genes from chromosome 11
- Someone who has parents or siblings diagnosed with Paris Trousseau
Platelets
The treatment for Paris Trousseau Syndrome is a platelet infusion. A platelet infusion is a transfusion of platelets, donated from another person, which is given in a vein. These infusions will provide platelets that work correctly and will help the body to stop bleeding. Mostly, people diagnosed with Paris Trousseau Syndrome only need treatment when injury occurs, before more invasive dental work and before surgeries.
Amicar/Lysteda (Aminocaproic Acid/Tranexamic Acid)
Amicar and Lysteda are oral medications that work very well for bleeding of mucous membranes such as nose and mouth bleeding. Lysteda is also used for heavy menstrual bleeding. When bleeding occurs, the body tries to heal the area by making a clot that will plug the hole to stop bleeding. This clot will stay there for about four days. After these four days, chemicals in the body are triggered to breakdown the clot. This is called fibrinolysis. Amicar and Lysteda are called anti-fibrinolytic medications because it stops fibrinolysis. A person with a bleeding disorder needs more time for healing because they cannot make a strong clot to heal the area of bleeding. Amicar and Lysteda stops the body from breaking the clot down. This gives the blood vessels more time to completely heal and prevents prolonged bleeding and rebleeding.
Bleeding can occur anywhere in the body. Usually bleeding is caused by injury, surgery or dental procedures.
Complications from Paris Trousseau Syndrome can include:
- Prolonged bleeding leading to anemia
- Bleeding in joints leading to arthritis or damage to joints
- Damage to the brain (Head injury resulting to bleeding inside the skull)
- Difficulty breathing (Due to bleeding in the neck or lungs after injury)
- Paralysis (Due to bleeding in the spinal column after injury
